Outcomes After Initial Unifocalization to a Shunt in Complex Tetralogy of Fallot With MAPCAs. Bauser-Heaton, H., Ma, M., Wise-Faberowski, L., Asija, R., Shek, J., Zhang, Y., Peng, L. F., Sidell, D. R., Hanley, F. L., & McElhinney, D. B. The Annals of Thoracic Surgery, 107(6):1807–1815, 2019.
doi  abstract   bibtex   
BACKGROUND: Our approach to tetralogy of Fallot (TOF) with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs) emphasizes early single-stage unifocalization and intracardiac repair. However, a subset of patients with small native pulmonary arteries (PAs) and MAPCAs undergo unifocalization to a shunt rather than simultaneous intracardiac repair. METHODS: Patients with TOF/MAPCAs who underwent complete unifocalization to a systemic-to-PA shunt by a single surgeon were reviewed. The decision to perform simultaneous intracardiac repair was based on an intraoperative flow study or empirical assessment of PA and MAPCA size. RESULTS: From November 2001 to December 2017, 57 patients with TOF/MAPCAs underwent unifocalization to a shunt at a median age of 6.9 months. Genetic abnormalities were documented in 60% of patients, including a chromosome 22q11 deletion in 25 and Alagille syndrome in 8. Twenty patients (35%) had undergone prior surgery elsewhere (n = 16) and/or at our center (n = 7). During a median follow-up of 5.4 years, 9 patients had additional surgery to revise the PA reconstruction before complete repair, and 38 patients underwent complete repair. Five years after unifocalization, survival was 74% ± 6%. At follow-up, the median PA to aortic systolic pressure ratio was 0.36 and was greater than 0.50 in 2 patients. CONCLUSIONS: In patients with the smallest MAPCAs and PAs, single-stage unifocalization to a shunt followed by intracardiac repair yields an excellent outcome in most cases.
@article{bauser-heaton_outcomes_2019,
	title = {Outcomes {After} {Initial} {Unifocalization} to a {Shunt} in {Complex} {Tetralogy} of {Fallot} {With} {MAPCAs}},
	volume = {107},
	issn = {1552-6259},
	doi = {10.1016/j.athoracsur.2019.01.030},
	abstract = {BACKGROUND: Our approach to tetralogy of Fallot (TOF) with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs) emphasizes early single-stage unifocalization and intracardiac repair. However, a subset of patients with small native pulmonary arteries (PAs) and MAPCAs undergo unifocalization to a shunt rather than simultaneous intracardiac repair.
METHODS: Patients with TOF/MAPCAs who underwent complete unifocalization to a systemic-to-PA shunt by a single surgeon were reviewed. The decision to perform simultaneous intracardiac repair was based on an intraoperative flow study or empirical assessment of PA and MAPCA size.
RESULTS: From November 2001 to December 2017, 57 patients with TOF/MAPCAs underwent unifocalization to a shunt at a median age of 6.9 months. Genetic abnormalities were documented in 60\% of patients, including a chromosome 22q11 deletion in 25 and Alagille syndrome in 8. Twenty patients (35\%) had undergone prior surgery elsewhere (n = 16) and/or at our center (n = 7). During a median follow-up of 5.4 years, 9 patients had additional surgery to revise the PA reconstruction before complete repair, and 38 patients underwent complete repair. Five years after unifocalization, survival was 74\% ± 6\%. At follow-up, the median PA to aortic systolic pressure ratio was 0.36 and was greater than 0.50 in 2 patients.
CONCLUSIONS: In patients with the smallest MAPCAs and PAs, single-stage unifocalization to a shunt followed by intracardiac repair yields an excellent outcome in most cases.},
	language = {eng},
	number = {6},
	journal = {The Annals of Thoracic Surgery},
	author = {Bauser-Heaton, Holly and Ma, Michael and Wise-Faberowski, Lisa and Asija, Ritu and Shek, Jennifer and Zhang, Yulin and Peng, Lynn F. and Sidell, Douglas R. and Hanley, Frank L. and McElhinney, Doff B.},
	year = {2019},
	pmid = {30772338},
	keywords = {Abnormalities, Multiple, Adolescent, Aorta, Thoracic, Cardiac Surgical Procedures, Child, Child, Preschool, Collateral Circulation, Female, Humans, Infant, Infant, Newborn, Male, Pulmonary Artery, Retrospective Studies, Tetralogy of Fallot, Treatment Outcome, Vascular Surgical Procedures},
	pages = {1807--1815}
}

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